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Klippel-Trenaunay syndrome or KTS is a complex vascular syndrome associated with overgrowth occurring as a result of somatic mutations in the PIK3CA gene. Patients are diagnosed on the basis of physical findings, sometimes with supportive imaging, of commonly a segmental anomaly with a cutaneous port-wine stain, lymphatic and venous malformations and overgrowth. The severity of the component vascular malformations and the degree of overgrowth varies from patient to patient which demands care given by a multi-professional team with regular follow-up in a specialist clinic. Some patients may present with acute life-threatening problems, often as a result of veno-thromboembolic events (VTEs) especially following surgical and invasive radiological procedures. Awareness of such problems is vital and prophylactic measures to reduce such risks are paramount. The interventional radiologist is vital to the care team as he/she can undertake procedures including endovascular closure of significant venous anomalies which predispose to such VTEs. Although these procedures can be lengthy and complex, they can now provide a minimally invasive means to reduce the risk from life-threatening and sometimes fatal VTEs. The results however from such interventions will require long-term studies which to date are unavailable., (Copyright © 2019. Published by Elsevier Inc.)

Background: Nontraumatic spontaneous subarachnoid hemorrhage (SAH) is associated with a high mortality. This study was conducted to investigate the epidemiological features of nontraumatic spontaneous SAH in China., Methods: From January 2006 to December 2008, the clinical data of patients with nontraumatic SAH from 32 major neurosurgical centers of China were evaluated. Emergent digital subtraction angiography (DSA) was performed for the diagnosis of SAH sources in the acute stage of SAH (≤3 days). The results and complications of emergent DSA were analyzed. Repeated DSA or computed tomography angiography (CTA) was suggested 2 weeks later if initial angiographic result was negative., Results: A total of 2562 patients were enrolled, including 81.4% of aneurysmal SAH and 18.6% of nonaneurysmal SAH. The total complication rate of emergent DSA was 3.9% without any mortality. Among the patients with aneurysmal SAH, 321 cases (15.4%) had multiple aneurysms, and a total of 2435 aneurysms were detected. The aneurysms mostly originated from the anterior communicating artery (30.1%), posterior communicating artery (28.7%), and middle cerebral artery (15.9%). Among the nonaneurysmal SAH cases, 76.5% (n = 365) had negative initial DSA, including 62 cases with peri-mesencephalic nonaneurysmal SAH (PNSAH). Repeated DSA or CTA was performed in 252 patients with negative initial DSA, including 45 PNSAH cases. Among them, the repeated angiographic results remained negative in 45 PNSAH cases, but 28 (13.5%) intracranial aneurysms were detected in the remaining 207 cases. In addition, brain arteriovenous malformation (AVM, 7.5%), Moyamoya disease (7.3%), stenosis or sclerosis of the cerebral artery (2.7%), and dural arteriovenous fistula or carotid cavernous fistula (2.3%) were the major causes of nonaneurysmal SAH., Conclusions: DSA can be performed safely for pathological diagnosis in the acute stage of SAH. Ruptured intracranial aneurysms, AVM, and Moyamoya disease are the major causes of SAH detected by emergent DSA in China.

Objective: Gamma knife radiosurgery (GKRS) has emerged as an important therapeutic alternative for different intracranial lesions. We have reviewed our institution's first 1,000 cases of radiosurgeries., Materials and Methods: We performed a retrospective review (2000-2013) of 1,017 radiosurgeries in 911 patients with various intracranial lesions including vestibular schwannoma (82), meningioma (136), metastatic brain tumors (298), astrocytoma (49), pituitary adenoma (92), arteriovenous malformation (85) and trigeminal neuralgia (169)., Results: GKRS in different intracranial lesions showed significant variations in outcome and complications. Overall, the local tumor growth control for benign and malignant tumors was 89 percent and 70 percent respectively. The rate of obliteration of arteriovenous malformation nidus was 79 percent. The complete and partial relief of pain in the patients with trigeminal neuralgia was 55.6 percent and 22.4 percent respectively., Conclusion: At recent follow-up, GKRS showed good control of different tumor growth, obliteration of AVM nidus and remission of trigeminal neuralgia pain, good overall and progression free survival rate, possible preservation of neurological functions, lesser number of complications, and improvement of quality of life. Therefore, GKRS is an important treatment option for patients with different benign intracranial tumors, AVM and trigeminal neuralgia. However, GKRS is not effective for recurrent malignant tumors in the brain.

Objective: To investigate predictive factors of complete obliteration following treatment with linac-based stereotactic radiosurgery for intracerebral arteriovenous malformations., Methods: Archived plans for 48 patients treated at the British Columbia Cancer Agency and who underwent post-treatment digital subtraction angiography to assess obliteration were studied. Actuarial estimates of obliteration were calculated using the Kaplan-Meier method. Univariate and multivariate Cox proportional hazards models were used for analysis of incidence of obliteration. Log-rank test was used to search for parameters associated with obliteration., Results: Complete nidus obliteration was achieved in 38/48 patients (79.2%). Actuarial rate of obliteration was 75.9% at 4 years (95% confidence interval 63.1%-88.6%). On univariate analysis, prescribed dose to the margin (p=0.002) and dose to isocentre (p=0.022) showed statistical significance. No parameters were significant in a multivariate model. According to the log-rank test, prescribed dose to the margin of >20 Gy (p=0.004) and dose to the isocentre of >25 Gy (p=0.004) were associated with obliteration., Conclusion: Reported series in the literature suggest a number of different factors are predictive of complete obliteration of arteriovenous malformations following radiosurgery. However, differing definitions of volume and complete obliteration makes direct comparison between series difficult. This study demonstrates that complete obliteration of the nidus following linear accelerator-based stereotactic radiosurgery for arteriovenous malformations appears to be most closely related to the prescribed marginal dose. In particular, a marginal dose of >20 Gy is strongly associated with obtaining complete obliteration of the nidus.

Purpose: To evaluate the prognosis of mortality in patients with spontaneous subarachnoid haemorrhage associated with Terson's syndrome., Methods: A prospective, consecutive case series study was conducted in patients admitted to the emergency room with a diagnosis of acute subarachnoid haemorrhage. After a complete neurological examination, funduscopic examination using binocular indirect ophthalmoscopy under mydriasis was performed upon admission and at days 3, 7, 30 and 60 after the onset. In all cases, the diagnosis of intracranial bleeding was made by computerized tomography, and the clinical condition was graded according to the Hunt & Hess and Glasgow coma scales., Results: Forty-seven patients with the diagnosis of subarachnoid haemorrhage were enrolled. Forty-four cases were associated with a ruptured aneurysm and three cases with arterio-venous malformation. Fourteen patients (29%) were diagnosed with Terson's syndrome. Seven patients (50%) with Terson's syndrome died, whereas death occurred in three patients (9%) without Terson's syndrome (p = 0.002). Ocular findings in Terson's syndrome were preretinal, intraretinal, sub-retinal and vitreous haemorrhage. Associated ocular findings included third-nerve palsy, papilloedema and subconjunctival haemorrhage., Conclusion: The presence of Terson's syndrome was associated with an increased mortality rate (50% versus 9%; p < 0.01). Therefore, patients with the diagnosis of intracranial haemorrhage should be submitted to a funduscopic examination, because the presence of intraocular haemorrhage is an important life-threatening prognostic factor., (© 2009 The Authors. Journal compilation © 2009 Acta Ophthalmol.)

Background: Hereditary hemorrhagic telangiectasia is a genetic disease characterized by teleangiectasias involving virtually every organ. There are limited data in the literature regarding the natural history of liver vascular malformations in hemorrhagic telangiectasia and their associated morbidity and mortality., Aim: This prospective cohort study sought to assess the outcome of liver involvement in hereditary hemorrhagic telangiectasia patients., Methods: We analyzed 16 years of surveillance data from a tertiary hereditary hemorrhagic telangiectasia referral center in Italy. We considered for inclusion in this study 502 consecutive Italian patients at risk of hereditary hemorrhagic telangiectasia who presented at the hereditary hemorrhagic telangiectasia referral center and underwent a multidisciplinary screening protocol for the diagnosis of hereditary hemorrhagic telangiectasia. Of the 502 individuals assessed in the center, 154 had hepatic vascular malformations and were the subject of the study; 198 patients with hereditary hemorrhagic telangiectasia and without hepatic vascular malformations were the controls. Additionally, we report the response to treatment of patients with complicated hepatic vascular malformations., Results: The 154 patients were included and followed for a median period of 44 months (range 12-181); of these, eight (5.2%) died from VM-related complications and 39 (25.3%) experienced complications. The average incidence rates of death and complications were 1.1 and 3.6 per 100 person-years, respectively. The median overall survival and event-free survival after diagnosis were 175 and 90 months, respectively. The rate of complete response to therapy was 63%., Conclusions: This study shows that substantial morbidity and mortality are associated with liver vascular malformations in hereditary hemorrhagic telangiectasia patients.

Introduction: We describe indications, outcomes, and risk profiles of fractionated stereotactic radiotherapy (SRT) and single fraction "radiosurgery" (SRS) in pediatric patients compared to the adult population and evaluate the causal role of SRS and SRT in inducing new neurological complications., Materials and Methods: Six children with AVMs and 12 children with neoplastic diseases were prospectively followed for >2 years after SRT/S. The survival, control of pathology, and specified neurological complications were analyzed. In tumor patients, the median overall survival time was 45 months (range 5-103) and the median progression free survival time was 35 months (range 5-98)., Results: Control or regression of the tumor was obtained in 83% of patients with neoplastic disease. Three patients with malignant tumors died from disease progression. In AVMs the median time follow up was 52 months (range 27-100). All AVMs were obliterated. New neurological deficits occurred in 67%. SRT/S was considered the direct cause in 25%. All the neurological deficiencies related to SRT/S were focal and related to the irradiated areas. In tumor patients, midline lesions, malignant diagnosis, and additional treatment with surgery, chemotherapy, and craniospinal irradiation seemed to increase the risk of new deficits after SRT/S. In AVM patients, a high Spetzler-Martin grade seemed to carry a higher complication risk., Conclusion: The risk of uncontrolled tumor disease or the risk of hemorrhage of non-obliterated AVM must be balanced against the overall risks and benefits of SRT/S. Following SRT/S, the risk of worsening pre-existing deficits is relatively high. The risk of inducing new long-term deficits is relatively low.

Hepatic involvement occurs in up to 74% of patients with hereditary hemorrhagic telangiectasia (HHT) and is characterized by a spectrum of arteriovenous malformations. Three different types of intrahepatic shunting may be present: hepatic artery to hepatic veins, hepatic artery to portal vein, and portal vein to hepatic vein. Hepatic involvement in HHT may lead to biliary ischemia, portal hypertension, or high-output cardiac failure (HOCF). Orthotopic liver transplantation (OLT) has been proposed as the only definitive curative treatment. The aim of this study was to evaluate the long-term outcome of patients with hepatic involvement due to HHT after OLT with respect to mortality, cardiac and hepatic status, epistaxis, and quality of life. Patients with HHT and severe hepatic vascular malformations who underwent OLT in the Lyon Liver Transplant Unit (LLTU) from 1993 to 2007 were followed at the LLTU and the French Reference Center for HHT. Quality of life was evaluated with the Short Form 36 questionnaire. There were 13 patients who fulfilled the entry criteria of the study (12 women and 1 man). The mean age at the time of OLT was 51.8 years (range = 33-65 years). Indications for OLT were cardiac failure (n = 9), biliary necrosis (n = 2), both cardiac failure and biliary necrosis (n = 1), and hemobilia (n = 1). The mean duration of follow-up was 109 months (range = 1-200 months). Twelve patients (92.3%) are still alive. For the 9 patients with HOCF, the mean cardiac index decreased from 5.4 L/minute/m(2) before OLT to 3.0 L/minute/m(2) after OLT. No severe hepatic complications were observed after OLT. Nine of the surviving patients (75%) experienced dramatic improvements in epistaxis and quality of life, including an ability to undertake more physical activity. In conclusion, OLT is an important therapeutic option for patients with HHT who have severe hepatic involvement. In the reported cohort, the mortality after OLT for this indication was low.

Brain arteriovenous malformations (AVMs) are highly complex vascular lesions, which may become symptomatic by causing hemorrhagic stroke, epilepsy, chronic headache and/or focal neurological deficits. An increasing number of AVMs are also found incidentally in MRI and CT scans performed for other reasons. It is mandatory to understand the natural history and prognosis of these malformations when evaluating the risks that may be associated with the treatment. In this review, we discuss the symptomatology, hemorrhage risk and mortality associated with their natural course.

Spontaneous (non-traumatic) intracerebral haemorrhage (ICH) has a high case-fatality and leaves many survivors disabled. Clinical characteristics and outcome seem to vary according to the cause of ICH, but population-based comparisons are scarce. We studied two prospective, population-based cohorts to determine differences in outcome [case-fatality and modified Rankin Scale (mRS)] after incident ICH due to brain arteriovenous malformations (AVM) [Scottish Intracranial Vascular Malformation Study (SIVMS), n = 90] and spontaneous ICH [Oxford Vascular Study (OXVASC), n = 60]. Patients with AVM-ICH were younger, had lower pre-stroke and admission blood pressure (BP), higher admission Glasgow Coma Scale (GCS) and were more likely to have an ICH in a lobar location than patients with spontaneous ICH (sICH). Case fatality throughout 2-year follow-up was greater following sICH than AVM-ICH [34/56 (61%) versus 11/90 (12%) at 1 year, odds ratio (OR) 11 (95% Confidence Interval (CI) 5-25)], as was death or dependence (mRS >or= 3) [40/48 (83%) versus 26/65 (40%) at 1 year, OR 8 (3-19)]. Differences in outcome persisted following stratification by age and sensitivity analyses. In multivariable analyses of 1 year outcome, independent predictors of death were sICH (OR 21, 4-104) and increasing ICH volume (OR 1.03, 1.01-1.05), and independent predictors of death or dependence were sICH (OR 11, 2-62) and GCS on admission (OR 0.79, 0.67-0.93). Outcome after AVM-ICH is better than after sICH, independent of patient age and other known predictors of ICH outcome.

The treatment of acute cerebrovascular disease plays a prominent role in clinical routine. However, prophylactic therapy of cerebrovascular disease including endovascular options is becoming more and more important. With particular regard to endovascular therapies, this summary deals with the natural course of cerebrovascular diseases and different treatment strategies for asymptomatic intracranial aneurysms, cerebral arteriovenous malformations, and extracranial carotid artery stenoses.

Objectives: Hereditary haemorrhagic telangiectasia (HHT) affects 1 in 5-8000 individuals. Pregnancy outcomes are rarely reported. The major reason is that most women do not have their HHT diagnosed prior to pregnancy. Using a large well-characterised series, we studied all pregnancies known to have occurred in HHT-affected women, whether or not their diagnosis was known at the time of pregnancy. Our aim was to estimate rates and types of major complications of HHT in pregnancy, to guide management decisions., Design: Cohort study, with prospective, retrospective and familial components., Setting/population: Tertiary referral centre population., Methods: All 262 pregnancies in the 111 women with HHT and pulmonary arteriovenous malformations (PAVMs) reviewed between 1999 and 2005 were studied. Eighty-two women (74%) did not have a diagnosis of HHT/PAVM at the time of pregnancy. 222 pregnancies in their 86 HHT-affected relatives were also studied., Main Outcome Measures: PAVM bleed, stroke and maternal death., Results: Thirteen women experienced life-threatening events during pregnancy: 1.0% (95% CI 0.1-1.9) of pregnancies resulted in a major PAVM bleed; 1.2% (0.3-2.2%) in stroke (not all were HHT related); and 1.0% (0.13-1.9%) in maternal death. All deaths occurred in women previously considered well. In women experiencing a life-threatening event, prior awareness of HHT or PAVM diagnosis was associated with improved survival (P = 0.041, Fisher's exact test)., Conclusions: Most HHT pregnancies proceed normally. Rare major complications, and improved survival outcome following prior recognition, means that pregnancy in a woman with HHT should be considered high risk. Recommendations for pregnancy management are provided.

Long-term follow-up results after embolization of 13 pulmonary arteriovenous malformations in 10 patients by use of 14 detachable silicone balloons are given. Patients were followed for a mean of 99 months (range, 63-123 months) with chest x-rays and for a mean of 62 months (range, 3-101 months) with pulmonary angiography. Fifty-four percent of the balloons were deflated at latest radiographic chest film follow-up, but at pulmonary angiographic follow-up all embolized malformations were without flow irrespective of whether or not the balloons were visible. Detachable silicone balloons are not available anymore, but use of these balloons for embolization of pulmonary arteriovenous malformations has been shown to be a safe and precise method, with immediate occlusion of the feeding artery and with long-lasting occlusion, even though many balloons deflate with time, leaving a fibrotic scar replacing the pulmonary arteriovenous malformation. No case of recanalization has been discovered, and these results seem to justify a reduced number of controls of these balloon-embolized malformations.

Objectives: To report our experience using embolization in managing localized pulmonary arteriovenous malformations in adults., Material: and methods. All patients presenting with localized pulmonary arteriovenous malformations treated with embolization were included in the study. Clinical presentation (respiratory symptoms and previous history of paradoxical embolism) and the characteristics of pulmonary arteriovenous malformations (single or multiple, location, diameter of the afferent artery and simple or complex angioarchitecture) before embolization were analyzed. The details of the procedure, including the number of pulmonary arteriovenous malformations embolized, the number of coils used, and the type of intraoperative complications were recorded. Postembolization clinical and imaging follow-up were described., Results: Forty-two patients (26 women, 16 men; mean age, 45 years), including 36 with hereditary hemorrhagic telangiectasia were treated with embolization. Twenty-two patients (53%) were dyspneic and 12 (29%) had a previous history of paradoxical embolism prior to embolization. Forty-seven procedures were carried out on a total of 99 pulmonary arteriovenous malformations (mean, 2.3 per patient), using 530 coils (12.6 per patient). The pulmonary arteriovenous malformations were located in the lower lobes in 60% of cases and a simple architecture was reported in 81% of cases. The average diameter of the afferent artery was 6mm. No preoperative complications were reported. After embolization, two patients (5%) presented with a paradoxical embolism and five patients out of 22 (23%) remained dyspneic. The rate of complete occlusion of treated arteriovenous malformations was 92% using computer tomography., Conclusion: Embolization is a highly effective and safe technique for treating pulmonary arteriovenous malformations. Improvement in dyspnea and prevention of paradoxical embolism can be expected. A high technical success rate can be obtained by experienced interventional radiologists.

Conclusions: For extracranial arteriovenous malformations of the head and neck (HNAVMs), in which the nidus was accessible via the percutaneous route, ethanol sclerotherapy was a feasible and safe first-line treatment, although successful outcomes were obtained for only about half of the subjects. For other HNAVMs, surgical excision with embolization may be the best choice of treatment., Objective: To suggest a treatment protocol for patients with HNAVMs by comparing the treatment outcomes and complications of ethanol sclerotherapy with those of surgical excision combined with embolization., Material and Methods: Twenty patients who had been diagnosed with HNAVM and treated between 1995 and 2002 were retrospectively reviewed. Ethanol sclerotherapy, surgical excision and embolization were used as treatments, either alone or in various combinations. The treatment outcomes and complications with the different modalities were analyzed., Results: Ethanol sclerotherapy was used for 12 cases, with a success rate of 50.0% and a permanent complication rate of 8.3%. Surgical excision combined with embolization was used for 13 patients. Although all patients achieved successful resolution of their HNAVM after surgical excision, 15.4% suffered from permanent complications. In total, 16/20 patients (80.0%) eventually achieved a > or = 75% reduction in the size of their lesions.

Vascular malformation (AVM) in the gastrointestinal tract is an uncommon, but not rare, cause of bleeding and iron deficiency anemia, especially in an aging population. While endoscopic coagulative therapy is the method of choice for controlling bleeding, a substantial number of cases require additional therapy. Adjunctive or even primary phamacotherapy may be indicated in recurrent bleeding. However, there is little evidence-based proof of efficacy for any agent. The bulk of support is derived from anecdotal reports or case series. The present review compares the outcome of AVM after no intervention, coagulative therapy or focus on pharmacological agents. Most of the literature encompasses two common AVMs, angiodysplasia and hereditary hemorrhagic telangiectasia. Similarly, the bulk of information evaluates two therapies, hormones (estrogen and progesterone) and the somatostatin analogue octreotide. Of these, the former is the only therapy evaluated in randomized trials, and the results are conflicting without clear guidelines. The latter therapy has been reported only as case reports and case series without prospective trials. In addition, other anecdotally used medications are discussed.

Background: In patients with polysplenia syndrome and azygous continuation of an interrupted inferior vena cava (IVC), pulmonary arteriovenous malformations (PAVMs) are relatively common after bidirectional cavopulmonary anastomosis (BCPA, Kawashima procedure). Resolution of PAVMs after hepatic vein (HV) inclusion into the cavopulmonary circulation has been reported, but there has been no systematic investigation of the effects of this therapy in a population of more than 3 patients., Methods: We studied 16 patients with heterotaxy, univentricular congenital heart disease, and azygous continuation of the IVC who underwent incorporation of the HV into the cavopulmonary circuit for treatment of significant PAVMs after a Kawashima procedure., Results: The median preoperative systemic arterial oxygen saturation (SsaO2) was 76% (65%-85%), compared with 89% (85% to 92%) early after BCPA. Among 15 early survivors, the median early postoperative SsaO2 was 76% (56%-85%). In 11 of the 15 survivors, SsaO2 rose to 90% or greater within a year and remained at 93% or greater at follow-up of 2.8 to 10 years. Four patients had persistent hypoxemia and residual PAVMs at follow-up catheterization 1.5 to 8 years postoperatively; these patients had the most severe hypoxemia prior to HV inclusion, and in 2 the residual PAVMs were unilateral, with HV flow streaming to the contralateral lung, in which PAVMs had resolved., Conclusions: Hypoxemia resolved after cavopulmonary incorporation of the HV in the majority of our patients with PAVMs after the Kawashima operation, presumably due to a combination of PAVM resolution and elimination of hepatic venoatrial right-to-left shunting. These findings support the theory that development of PAVMs is facilitated by exclusion of HV effluent from the pulmonary circulation.

Background: Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease, characterized by a wide variety of clinical manifestations, including epistaxis, gastrointestinal (GI) bleeding, pulmonary arteriovenous malformations (PAVMs) and neurological symptoms. HHT is a genetically heterogeneous disorder involving at least two loci; HHT 1 mapping to chromosome 9 q 34.1 (ENG) and HHT 2 mapping to chromosome 12 q 31 (ALK-1)., Objective: To evaluate and describe the diversity of clinical manifestations in a Danish population of HHT patients with known HHT 1 or HHT 2 subtype., Design: Prospective clinical examination with genetic evaluation and follow-up., Setting: Investigation centre was Odense University Hospital. All HHT patients in the County of Fyn were included., Methods: HHT family members were invited to a clinical examination including registration of HHT manifestations, screening for PAVM and neurological evaluation. Blood tests were performed for analysis of disease-causing mutation, and clinical manifestations in the HHT subtypes were compared. The survival of the patients was studied in the follow-up period., Results: Included in the study were 73 HHT patients representing 18 families. In 14 of the families we identified a disease-causing mutation. Thirty-nine patients (from 10 families) had HHT1 and 16 HHT patients from four families had HHT2., Conclusion: Amongst patients with HHT1 genotype the prevalence of PAVM was higher than amongst HHT patients with HHT2 genotype. HHT1 patients had experienced more severe GI bleeding than HHT2 patients. There was no significant difference in severity of epistaxis or age at debut. Finally the mortality over a 90-month observation period was not significantly increased.

Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels. Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber-Osler-Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere. They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure. Cerebral vascular accidents are also a well-known complication of this disorder. An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber-Osler-Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome. These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.

Background and Study Aims: Dieulafoy lesion is a rare cause of massive gastrointestinal hemorrhage, most commonly from the proximal stomach. Surgery was regarded as the treatment of choice in the past, but recently endoscopic management has become the standard approach. However, the effectiveness of various endoscopic modalities in treating bleeding Dieulafoy lesion has been little studied. This study was therefore done to compare the hemostatic efficacy of endoscopic hemoclipping and epinephrine injection therapy., Patients and Methods: Between January 1998 and December 2001, a consecutive series of 32 patients with bleeding Dieulafoy lesion underwent endoscopic treatment. They were randomly treated either by endoscopic epinephrine injection therapy (n = 16) or by hemoclipping (n = 16). We compared mortality rate, primary hemostasis rate, and rebleeding rate between two groups., Results: There was no bleeding-related death in either group. There was no significant difference in primary hemostasis rates between the hemoclipping group (93.8 %) and epinephrine injection group (87.5 %, P = 1.00). There was a trend toward a lower rate of need for multiple endoscopic sessions to achieve permanent hemostasis in the hemoclipping group compared with the epinephrine injection group (6.3 % vs. 31.3 %, P = 0.086). Hemoclipping was significantly more effective in preventing recurrent bleeding than epinephrine injection therapy (0 % vs. 35.7 %, P < 0.05). With regard to lesion site, hemoclipping was significantly more effective in preventing recurrent bleeding of gastric body Dieulafoy lesion than epinephrine injection therapy (0 % vs. 50 %, P < 0.05)., Conclusions: Bleeding from Dieulafoy lesion was well controlled by therapeutic endoscopic procedures. Hemoclipping was more effective for Dieulafoy lesion than epinephrine injection therapy, with less need for subsequent endoscopy.

Background: Dieulafoy's lesion is a rare but important cause of upper gastrointestinal bleeding. Current endoscopic methods used to treat Dieulafoy's lesion include injection, with or without thermal methods, and mechanical methods. The latter include variceal ligation and hemoclips. There are no studies comparing the outcomes of rubber band ligation and injection with or without thermal therapy., Aim: To report the outcomes of Dieulafoy's lesion treated endoscopically with rubber band ligation and injection with or without thermal therapy at a single institution., Methods: Patients with the diagnosis of Dieulafoy's lesion treated endoscopically at the Carl T. Hayden VA Medical Center in Phoenix, between August 1994 and August 2002 were analyzed. Demographic data, mode of presentation, risk factors for gastrointestinal bleeding, hemodynamic parameters, blood transfusion requirements, endoscopic findings, details of endoscopic therapy, length of stay in ICU/hospital, complications, recurrence of bleeding, and mortality rates were collected and compared between those receiving endoscopic band ligation (EBL group) and those receiving injection with or without thermal therapy (non-EBL group)., Results: Twenty-three patients with Dieulafoy's lesion (14 in the EBL group and nine in the non-EBL group) were studied. All patients were men. The mean age, hemoglobin levels on admission, and the transfusion requirements before therapy were similar in both groups. Fourteen patients (eight in the EBL- and six in the non-EBL groups) presented with hematemesis and the remaining with melena. The majority of Dieulafoy's lesions (91.3%) were located in the stomach and two in the duodenum. Active bleeding at the time of endoscopy was seen in 61% of cases, and immediate hemostasis was achieved with either method in 100% of patients. Early rebleeding (within 72 hours of endoscopic therapy) occurred in only one patient treated with epinephrine plus heater probe therapy. The length of stay in ICU was longer in the non-EBL group (6.7 days) compared with the EBL group (1.8 days) (P = 0.2). There were six deaths (three in the non-EBL group and three in the EBL group) within 30 days of the index hospitalization. The causes of death included infection/sepsis (n = 3), complications of acute myocardial infarction (n = 2), and end-stage liver disease (n = 1)., Conclusions: Endoscopic rubber band ligation is as effective as injection with or without thermal therapy in the treatment of Dieulafoy's lesion.

Background: The Dieulafoy lesion is an important cause of gastrointestinal (GI) hemorrhage. Optimal treatment and long-term outcome are unknown. This study aimed to characterize the presentation of the Dieulafoy lesion and to summarize the results and report the long-term outcome of endoscopic therapy., Methods: Data regarding diagnosis, treatment and outcomes were derived from our GI Bleed Team database, patient records and follow-up correspondence., Results: Ninety Dieulafoy lesions were identified in 89 patients after a mean of 1.9 endoscopies. Their mean age was 72 years. Thirty-four percent of lesions were extragastric. Median transfusion requirement was 5 units. Two patients exsanguinated and 3 required surgery; all others were initially successfully treated endoscopically (with or without epinephrine injection): heat probe (71 patients), band ligation (3), hemoclip (1), laser (2), bipolar probe (4), sclerotherapy (2) and epinephrine alone (2). Gastric perforation occurred in 1 patient following sclerotherapy. Thirty-day mortality was 13%, 4 related to hemorrhage and 5 related to comorbidity. During median follow-up of 17 months, 34 patients (42%) died. One patient had recurrent bleeding 6 years after operation., Conclusions: Dieulafoy lesion is relatively common and often extragastric. Endoscopic therapy is safe and effective. Long-term recurrence was not evident following endoscopic ablation. Follow-up after ablative therapy appears unnecessary.

The authors analyse their diagnostic and therapeutic experience with 26 patients suffering from congenital pulmonary arteriovenous aneurysms. Symptoms of the disease were found to depend directly on the presence and volume of arterial blood shunted into the venous bed. The volume of shunted nonoxygenated blood varied between 20 and 48 p.c.; the reflux caused hypoxic hypoxaemia with HbO2art. = 91.2 +/- 1.43%; pO2art. = 63.6 +/- 5.54 mmHg. Aimed tomography and pulmonary angiography proved most advantageous in terms of diagnostic; with their aid it was possible to identify three variants of congenital pulmonary arteriovenous aneurysm: isolated, multiple and "of the capillary haemangioma type".

Overwhelming and confusing data regarding the natural history of arteriovenous malformations can confound physicians attempting to advise patients whether surgery of unruptured arteriovenous malformations is indicated. Decision analysis is a new mathematical tool that allows physicians to compare options in such patients. With the use of a simple office computer, a software program was devised to compare surgical versus conservative options in a hypothetical 25-year-old man. The computer model weighs the important considerations of patient age, mortality and morbidity rates of the physician performing the surgery, rebleeding from the arteriovenous malformation, and patient desires, using the Markov process. When mortality and morbidity rates are reasonable, the mathematical process supports surgical intervention in the hypothetical patient. Patient guidelines for older patients and future plans for the model using grading systems are discussed.

Thirteen new patients and 174 patients previously reported with coronary arteriovenous fistula (CAVF) were reviewed to delineate the course and management of CAVF and to clarify the role of surgical ligation in the young asymptomatic patient. Patients were grouped according to age: 99 patients (four new and 95 reported) were less than 20 years old and 88 (nine new and 79 reported) were greater than or equal to 20 years old. Of those under 20 years of age, 19% had preoperative symptoms or CAVF-related complications, including congestive heart failure (CHF) in 6%, subacute bacterial endocarditis in 3% and death in one patient. Seventy-six patients younger than 20 years old had CAVF ligation with only one significant complication. In contrast, 63% of the older group and all of our nine older patients had preoperative symptoms or complications, including CHF in 19%, SBE in 4%, myocardial infarction (MI) in 9%, death in 14% and fistula rupture in one patient. Of the 43 ligated older patients, 23% had surgical complications, including MI in three and death in three. Mean pulmonic-to-systemic flow in the entire group was 1.6:1 and did not differ significantly in those with or without symptoms or complications. One of our patients and one previously reported had spontaneous CAVF closure. In summary, early elective ligation of CAVF is indicated in all patients because of the high incidence of late symptoms and complications and the increased morbidity and mortality associated with ligation in older patients.

The authors relate their experience concerning the surgical correction of congenital coronary fistula. Between May 1971 and June 1986, 9 patients (4 boys and 5 girls) aged from 17 days to 49 years were operated upon at the Division of Cardiac Surgery of Bergamo (Ospedali Riuniti). All the patients, except three who were asymptomatic, showed early cardiac failure or dyspnoea on effort and angina in the elderly. At the physical examination a continuous murmur was heard in 8 cases; the chest x-ray showed significant cardiomegaly and on the electrocardiogram a right/left ventricular hypertrophy pattern was detected in 5 patients. All the patients underwent cardiac catheterization and a coronary angiography. The left-to-right shunt ranged from 60% to 250% of the cardiac output. The anomalous communication affected the right coronary artery in 7 cases and the left in 2, opening into the right atrium in 4 patients, into the right ventricle in 3 and into the pulmonary artery in 2. All patients but one, in whom division and suture were the only necessary procedures, underwent correction by means conventional cardiopulmonary by-pass with moderate hypothermia. In 3 cases closure through the coronary artery was preferred, in 1 through the right ventricle and in 2 transpulmonary. There was only 1 late death which occurred in a 3 year-old patient due to renal failure. After a mean follow-up of 6 years, 7 patients are to be asymptomatic while 1 patient had to be reoperated for a significant residual shunt.

Twelve patients suffering from haemorrhages from a superficial exulceration (Dieulafoy's disease) were treated on an emergency basis during the period from 1981 to 1987. Preoperative diagnosis by means of gastroscopy was possible in 3 cases only. The sites of predilection within the cardia and near to it, except the curvatures, determined the surgical access by means of high gastrotomy-independent of a verification of the diagnosis. In 11 cases the therapy consisted of the purse-string ligature, in one case endoscopic sclerosing was successful. The mortality rate was 16.6%.

Although numerous reports are available concerning infants who have single umbilical artery, there are no studies that correlate the incidence of congenital malformations or prognosis of such infants with the presence or absence of an atrophied artery in their associated umbilical cords. For this reason, we studied 48 infants who have single umbilical artery. No significant differences were detected in congenital malformations or neonatal mortality between infants with atrophy of one of their arteries and infants with arterial aplasia.